原发性IgA肾病伴弥漫性新月体形成的临床特点及预后分析

Clinical characteristics and prognostic analysis of primary IgA nephropathy with diffuse crescent formation

目的了解弥漫性新月体IgA肾病(CIgAN)患者的临床病理特征和预后。方法2007年7月至2022年7月经肾活检确诊为弥漫性新月体IgAN患者33例,分析临床及病理资料。随访时间超过6个月患者22例纳入随访队列。结果33例患者中男18例(55%),女15例(45%),肾活检时年龄为36(21.5,51)岁,血清肌酐(Scr)153(92,271)μmol/L,新月体百分比为62.5%(54.2%,66.7%),其中细胞纤维性新月体为(26.1%±16.5%)。13例患者有前驱呼吸道感染。22例患者平均随访时间为25(8,72)个月。10例(46%)患者进展至复合肾脏结局,其中8例(36%)发展为终末期肾脏病。多因素Cox回归结果显示,肾活检时Scr是复合肾脏结局的独立危险因素,而新月体的百分比与复合肾脏结局无关。受试者工作特征曲线结果表明,即使接受免疫抑制剂治疗,肾活检时Scr浓度大于149μmol/L的弥漫性新月体IgAN患者预后不良风险仍然较高。结论弥漫性新月体IgAN病情进展快,预后差,尤其是肾活检时Scr浓度大于149μmol/L的患者。新月体类型以细胞纤维性新月体为主。肾活检时血清Scr浓度是弥漫性新月体IgAN患者eGFR下降≥50%或ESKD的独立危险因素,而新月体的百分比与患者预后无明显相关性。

Objective To understand the clinical and pathological characteristics and prognosis of patients with diffuse crescentic IgA nephropathy(CIgAN).Methods Thirty-three patients diagnosed as CIgAN through kidney biopsy from July 2007 to July 2022 were selected.Their clinical and pathological data were analyzed.Twenty-two patients followed-up for more than 6 months were included in the follow-up cohort.Results In the 33 patients,there were 18 males(55%)and 15 females(45%).The age at the time of kidney biopsy was 36(21.551)years old and their serum creatinine(Scr)was 153(92271)μmol/L.Their percentage of crescents was 62.5%(54.2%66.7%),with cellular fibrocellular crescents accounting for 26.1±16.5%.Thirteen patients had antecedent upper respiratory tract infections.The average follow-up time was 25(872)months in the follow-up cohort.Of the 22 patients,10 patients(46%)progressed to composite renal outcomes,and 8(36%)developed end-stage kidney disease(ESKD).Multivariate Cox regression analysis showed that Scr at the time of kidney biopsy was an independent risk factor for composite renal outcomes,while the percentage of crescents was not associated with composite renal outcomes.The receiver operating characteristic curve(ROC)analysis indicated that even with immunosuppressive therapy,patients with diffuse crescentic IgA nephropathy and serum Scr concentration greater than 149μmol/L at the time of kidney biopsy still had a high risk of poor prognosis.Conclusions CIgAN tends to progress rapidly and has a poor prognosis,especially in patients with Scer concentration greater than 149μmol/L at the time of kidney biopsy.The predominant type of crescents is cellular fibrocellular crescents.Serum Scr concentration at the time of kidney biopsy is an independent risk factor for a decline in estimated glomerular filtration rate(eGFR)of≥50%or ESKD in patients with CIgAN.The percentage of crescents is not significantly correlated with patient prognosis.